HIBADH 抗体 (AA 251-336) (Alexa Fluor 647)
-
- 抗原 See all HIBADH 抗体
- HIBADH (3-hydroxyisobutyrate Dehydrogenase (HIBADH))
-
抗原表位
- AA 251-336
-
适用
- 人, 小鼠
-
宿主
- 兔
-
克隆类型
- 多克隆
-
标记
- This HIBADH antibody is conjugated to Alexa Fluor 647
-
应用范围
- Western Blotting (WB), Immunofluorescence (Cultured Cells) (IF (cc)), Immunofluorescence (Paraffin-embedded Sections) (IF (p))
- 交叉反应
- 人, 小鼠
- 预测反应
- Rat,Cow,Sheep,Horse
- 纯化方法
- Purified by Protein A.
- 免疫原
- KLH conjugated synthetic peptide derived from human HIBADH
- 亚型
- IgG
- Top Product
- Discover our top product HIBADH Primary Antibody
-
anti-3-hydroxyisobutyrate Dehydrogenase (HIBADH) (AA 1-336) antibody
HIBADH 适用: 人 WB, IF 宿主: 小鼠 Polyclonal unconjugated
anti-3-hydroxyisobutyrate Dehydrogenase (HIBADH) (AA 1-257) antibodyHIBADH 适用: 人, 小鼠, 大鼠 WB, IHC, IHC (p) 宿主: 兔 Polyclonal unconjugated
anti-3-hydroxyisobutyrate Dehydrogenase (HIBADH) (AA 128-336) antibodyHIBADH 适用: 人, 小鼠 WB, IHC, IHC (p) 宿主: 兔 Polyclonal unconjugated
anti-3-hydroxyisobutyrate Dehydrogenase (HIBADH) (AA 1-336) antibodyHIBADH 适用: 人 WB 宿主: 兔 Polyclonal unconjugated
anti-3-hydroxyisobutyrate Dehydrogenase (HIBADH) antibodyHIBADH 适用: 人 ELISA, IHC 宿主: 兔 Polyclonal unconjugated
anti-3-hydroxyisobutyrate Dehydrogenase (HIBADH) (AA 252-301) antibodyHIBADH 适用: 人, 小鼠, 大鼠, Cow, 兔, 犬, 马, 斑马鱼, 豚鼠, Bat, Hamster, 猴, Pig WB 宿主: 兔 Polyclonal unconjugated
anti-3-hydroxyisobutyrate Dehydrogenase (HIBADH) (AA 144-193) antibodyHIBADH 适用: 人, 小鼠, 大鼠, Cow, 兔, 犬, 马, Bat, Hamster, 猴, Pig, 非洲爪蟾 WB 宿主: 兔 Polyclonal unconjugated
anti-3-hydroxyisobutyrate Dehydrogenase (HIBADH) (C-Term) antibodyHIBADH 适用: 人, 小鼠, 大鼠, Cow WB, IHC 宿主: 兔 Polyclonal unconjugated
-
- 应用备注
-
IF(IHC-P) 1:50-200
IF(IHC-F) 1:50-200
IF(ICC) 1:50-200 - 限制
- 仅限研究用
-
- 状态
- Liquid
- 浓度
- 1 μg/μL
- 缓冲液
- Aqueous buffered solution containing 0.01M TBS ( pH 7.4) with 1 % BSA, 0.03 % Proclin300 and 50 % Glycerol.
- 储存液
- ProClin
- 注意事项
- This product contains ProClin: a POISONOUS AND HAZARDOUS SUBSTANCE, which should be handled by trained staff only.
- 储存条件
- -20 °C
- 储存方法
- Store at -20°C. Aliquot into multiple vials to avoid repeated freeze-thaw cycles.
- 有效期
- 12 months
-
- 抗原
- HIBADH (3-hydroxyisobutyrate Dehydrogenase (HIBADH))
- 别名
- Hibadh (HIBADH 产品)
- 别名
- NS5ATP1 antibody, 6430402H10Rik antibody, AI265272 antibody, hibadh antibody, zgc:66262 antibody, wu:fb07f02 antibody, zgc:100804 antibody, 3-hydroxyisobutyrate dehydrogenase antibody, 3-hydroxyisobutyrate dehydrogenase b antibody, 3-hydroxyisobutyrate dehydrogenase S homeolog antibody, 3-hydroxyisobutyrate dehydrogenase a antibody, HIBADH antibody, Hibadh antibody, hibadhb antibody, hibadh.S antibody, hibadha antibody
- 背景
-
Synonyms: 3 hydroxy 2 methylpropanoate:NAD+ oxidoreductase, 3 hydroxyisobutyrate dehydrogenase, 3 hydroxyisobutyrate dehydrogenase mitochondrial, EC 1.1.1.31, MGC40361, NS5ATP1, 3HIDH_HUMAN.
Background: HIBADH is a 336 amino acid mitochondrial enzyme that catalyzes the NAD+-dependent, reversible oxidization of 3-Hydroxyisobutyrate to methylmalonate semialdehyde, an intermediate of valine catabolism. The enzyme functions as a homodimer between a pH of 7.0 and 10.0, with optimal activity between 8.8 and 9.0. It was previously hypothesized that defects in the gene encoding HIBADH may be the cause of 3-Hydroxyisobutyric aciduria, a rare disorder that is characterized by a variety of clinical manifestations such as neurodevelopmental problems and dysmorphic features. However, it was shown that HIBADH activity was equal in patients with 3-Hydroxyisobutyric aciduria as compared with controls.
- 基因ID
- 11112
-
